Assessing short term effects of Orkambi on lung function in CF patients

FRI used to assess short-term effects of Orkambi on lung function in patients with CF homozygous for F508de

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator Orkambi (lumacaftor-ivacaftor) has shown modest benefits in previous research, but the exact effects in the CF lung remain unclear.

Functional Respiratory Imaging (FRI) provides detailed information about lung structure and function, allowing a more in-depth understanding of treatment effects.

Orkambi significantly decreased air trapping at expiration as well as airway wall volume, which can be related to improved mucociliary transport.

These results indicate that FRI parameters reflecting regional changes to lung structure are more sensitive to treatment effects of Orkambi than conventional spirometry.

This research has been presented as a poster (hyperlink) during the ATS 2020 Virtual Conference.

Categorised in: / September 25, 2020 10:33 am / Published by